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Eltrombopag to Treat Thrombocytopenia During Last Month of Pregnancy in a Woman With MYH9-Related Disease: A Case Report

Favier, Remi, MD*†; De Carne, Celine, MD; Elefant, Elisabeth, MD§; Lapusneanu, Ruxanda, MD*†; Gkalea, Vasiliki, MD; Rigouzzo, Agnès, MD

doi: 10.1213/XAA.0000000000000621
Case Reports: Case Report

MYH9-related disease (MYH9-RD) is an inherited rare autosomal dominant macrothrombocytopenia. Patients with MYH9-RD have giant platelets and leukocyte inclusion bodies caused by mutations in the MYH9 gene encoding the non–muscle myosin heavy chain II-A. Before identification of the causative gene, patients were diagnosed as Epstein or Fechtner or Sebastian syndromes or May-Hegglin anomaly. As with other inherited thrombocytopenias, the risk of increased bleeding during perioperative period or delivery is a major concern. We report here the first successful cesarean delivery of a woman with MYH9-RD treated with eltrombopag during the last month of pregnancy.

From the *Assistance Publique-Hôpitaux de Paris, Paris, France; French Reference Centre for Inherited Platelet Disorders, A Trousseau Children Hospital, Paris, France; Department of Obstetrics and Gynecology and §French Reference Centre for Teratogenic Agents, A Trousseau Children Hospital, Paris, France; Haematological Laboratory, Tenon Hospital, Paris, France; and Department of Anesthesiology, A Trousseau Children Hospital, Paris, France.

Accepted for publication June 27, 2017.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Agnès Rigouzzo, MD, Service d’Anesthésie, Hôpital d’enfants Armand Trousseau, AP-HP, 26 av du Dr Arnold Netter, 75571 Paris cedex 12, France. Address e-mail to

© 2018 International Anesthesia Research Society
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